Horner’s Syndrome and Contralateral Abducens Nerve Palsy Associated with Zoster Meningitis

Case Report pISSN: 1011-8942 eISSN: 2092-9382

Korean J Ophthalmol 2013;27(6):474-477 http://dx.doi.org/10.3341/kjo.2013.27.6.474

© 2013 The Korean Ophthalmological Society

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474

Horner’s Syndrome and Contralateral Abducens Nerve Palsy Associated with Zoster Meningitis

Bum-Joo Cho

, Ji-Soo Kim

, Jeong-Min Hwang

1

Department of Ophthalmology, Seoul National University College of Medicine, Seoul, Korea

2

Department of Neurology, Seoul National University Bundang Hospital, Seongnam, Korea

3

Department of Ophthalmology, Seoul National University Bundang Hospital, Seongnam, Korea

Horner’s syndrome, first described by Johann Friedrich Horner in 1929, is characterized by the classic triad of mi- osis, ipsilateral blepharoptosis, and facial anhidrosis [1]. It can be caused by a viral infection, trauma, carotid dissec- tion, or a tumor which interrupts the sympathetic innerva- tion to the eyes and ocular adnexae [1]. Although the syn- drome usually manifests by itself, it occasionally presents with other conditions such as ipsilateral abducens nerve palsy or herpes zoster ophthalmicus [2,3]. Many research- ers have described the concurrence of Horner’s syndrome and ipsilateral abducens nerve palsy [4-6], and the under- lying causes were primary or metastatic tumors in the cav-

ernous sinus, sphenodial sinus cyst, carotid aneurysm, or viral infections [2,3,7,8].

Herein, we describe a case of Horner’s syndrome associ- ated with contralateral abducens nerve palsy, which has not been previously reported. Diverse manifestations in- volving both eyes with other neurologic symptoms were caused by zoster meningitis and were successfully treated with anti-viral medication. To the best of our knowledge, this is the first description of this association.

Case Report

A 55-year-old woman presented with diplopia that had developed 12 days prior. The diplopia was preceded five days earlier by malaise and two days earlier by tingling sensation and pain in the right shoulder. The paresthesia spread along the whole right arm, and then arm weakness developed. Three days after the occurrence of paresthesia, the patient developed multiple painful vesicular eruptions A 55-year-old woman presented with diplopia following painful skin eruptions on the right upper extremity.

On presentation, she was found to have 35 prism diopters of esotropia and an abduction limitation in the left eye. Two weeks later, she developed blepharoptosis and anisocoria with a smaller pupil in the right eye, which increased in the darkness. Cerebrospinal fluid analysis showed pleocytosis and a positive result for immunoglobulin G antibody to varicella zoster virus. She was diagnosed to have zoster meningitis with Horner’s syndrome and contralateral abducens nerve palsy. After intravenous antiviral and steroid treatments, the vesicular eruptions and abducens nerve palsy improved. Horner’s syndrome and diplopia resolved after six months. Here we present the first report of Horner’s syndrome and contralateral abducens nerve palsy associated with zoster meningitis.

Key Words: Abducens nerve palsy, Horner syndrome, Zoster meningitis

Received: June 5, 2012 Accepted: September 14, 2012

Corresponding Author: Jeong-Min Hwang, MD. Department of Oph- thalmology, Seoul National University Bundang Hospital, #82 Gumi-ro 173beon-gil, Bundang-gu, Seongnam 463-707, Korea. Tel: 82-31-787-7372, Fax: 82-31-787-4057, E-mail: [email protected]

This paper was presented as a poster at the 98th annual meeting of Kore-

an Ophthalmological Society, Goyang, Korea, November 2007.

475 BJ Cho, et al. Horner Syndrome with Contralateral Abducens Palsy

on the right shoulder. After three more days, the tingling sensation involved the right lower leg.

On presentation, she was found to have 35 prism diop- ters of esotropia, which worsened on left gaze at distance and near in the primary position. There was an abduction limitation in the left eye (Fig. 1A). The pupils were isocoric and reactive in both eyes. Visual acuity, the margin-reflex distance, levator function of the eyelid, anterior segments and fundi were normal in both eyes. The ocular and medi- cal histories were unremarkable.

On neurological examination, right upper extremity strength was decreased to Medical Research Council grade

II. Electromyography revealed a right C4 and C5 radicu- lopathy. Sensation was also decreased in the right C3 to C5 dermatomes. Although mental status was intact and brain magnetic resonance imaging (MRI) was normal, an analy- sis of cerebrospinal fluid (CSF) showed pleocytosis of 26 cells/mm

and immunoglobulin (Ig) G varicella zoster vi- rus (VZV) antibody. IgG and IgM VZV antibodies were detected in the serum as well. The patient was diagnosed as having zoster meningitis and was admitted for antiviral treatment. Acyclovir was administered intravenously and analgesics by mouth. With treatment, the skin lesions and shoulder weakness improved within one week.

Fig. 1. (A) Left abducens nerve palsy in a 55-year-old woman with zoster meningitis. (B) Improved left abducens nerve palsy one month after hospital discharge.

A

B

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On the 14th day after admission, she newly developed right blepharoptosis. The marginal reflex distance 1 was 1.5 mm in the right eye and 3.5 mm in the left eye. The levator function was 12 mm in both eyes. The patient complained of hemifacial anhidrosis on the right side. Pupils were re- active in both eyes, however were anisocoric with the pupil size of 3 mm in the right eye and 4 mm in the left eye in light. Anisocoria increased in the darkness to 3 mm in the right eye and to 4.5 mm in the left eye (Fig. 2). She was di- agnosed as having Horner’s syndrome in the right eye.

There was no change in the abduction limitation of the left eye. By the 22nd day, the motor and sensory functions re- covered considerably and the patient was discharged.

When she returned to clinic one month after discharge, she demonstrated improvement of the abduction limitation in the left eye (Fig. 1B), but anisocoria and blepharoptosis were still persistent.

Six months after discharge, the abduction limitation and diplopia were completely restored. Anisocoria, blepharo- ptosis, and hemifacial anhidrosis resolved. The only re- maining symptom was a mild paresthesia of the right arm.

Discussion

When Horner’s syndrome is associated with ipsilateral sixth nerve palsy, the condition strongly implies a posterior cavernous sinus lesion [6,7]. However, the present case de- veloped a contralateral abducens nerve palsy associated with Horner’s syndrome, which has not been previously reported. Unlike the ipsilateral condition, it is not easy to explain the coexistence of Horner’s syndrome and a con-

tralateral abducens nerve palsy with a single localized le- sion because the pathways of the oculosympathetic nerve and the contralateral abducens nerve are distant from each other. The present case had additional multiple neuronal involvements including the sensory and motor systems, as well as meninges. Furthermore, the sensory symptoms presented in multiple dermatomes of both the upper and lower extremities. Thus, it is difficult to confine the lesion.

Among diseases causing multiple neurological disorders, we reached the diagnosis of zoster meningitis. The clinical manifestations of herpes zoster such as painful eruptions, paresthesia, and arm weakness were helpful clues. Diagno- sis was based on CSF pleocytosis and the presence of IgG VZV antibodies in CSF [9]. Brain MRI findings were nor- mal, but consistent with previous reports [10]. The thera- peutic effects of antiviral medication also supported the diagnosis.

When a patient presents with ophthalmoplegia, facial paralysis, and motor dysfunction, peripheral neuromuscu- lar diseases such as Guillain-Barre syndrome, particularly Miller Fisher syndrome, should be ruled out [11]. The diag- nosis of Miller Fisher syndrome was excluded for five rea- sons. Firstly, paresis of extraocular muscles in Miller Fish- er syndrome is typically bilateral and accompanied by ataxia and areflexia. However, our patient did not demon- strate areflexia or ataxia, and only the abduction was limit- ed unilaterally. Secondly, she developed right arm weak- ness accompanied by vesicular skin eruptions which also favors zoster meningitis rather than Miller Fisher syn- drome. Thirdly, ataxia in Miller Fisher syndrome consists of a lack of voluntary coordination of muscle movements, but the arm weakness in our patient was muscular paraly- sis. Fourthly, combined anhidrosis also implies sympathet- ic dysfunction of Horner’s syndrome. Lastly, CSF study re- vealed pleocytosis commonly seen in viral meningitis, not albuminocytological dissociation in Miller Fisher syn- drome. Therefore, Horner’s syndrome fits this case better than Miller Fisher syndrome.

The presence of the zoster meningitis explains the mech- anism of multiple neuronal involvements. In zoster menin- goencephalitis, VZV can reach various areas of the central nervous system through meninges and causes diffuse neu- ronal damage [12]. VZV may lead to clinical manifesta- tions such as headache, malaise, pyrexia, nerve palsies, paresthesia, hemiplegia, and even Horner’s syndrome [13].

Generally accepted pathogenesis of VZV includes a direct Fig. 2. Blepharoptosis and augmented anisocoria in the dark of

the right eye, which was associated with a right hemifacial anhi-

drosis.

477 BJ Cho, et al. Horner Syndrome with Contralateral Abducens Palsy

cytopathic effect on neural tissue, an allergic reaction of the nerve to the virus, and an occlusive vasculitis induced by the virus [13].

In conclusion, we suggest that viral infection should be considered with a high priority in the diagnosis of multiple neuronal dysfunctions. In particular, if neurological signs or symptoms manifest in both eyes, systemic as well as central nervous lesions should be ruled out. The common pathogen might be herpes simplex virus or VZV. The clin- ical findings of the present case may be helpful in diagnos- ing patients who present with several neuronal involve- ments.

Conflict of Interest

No potential conflict of interest relevant to this article was reported.

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